Isolated unilateral pulmonary artery atresia in an adult presenting with cor pulmonale.

Unilateral pulmonary artery atresia (UPAA) is a rare embryonic vascular malformation, leading to general presentations of exertional dyspnoea, pneumonia and haemoptysis. Our patient, a man in his early 30s, presented with a history of progressive breathlessness over a period of 2 years. History showed multiple admissions for pneumonia over his childhood and adolescence. Physical examination revealed a loud P2, an ejection systolic murmur and coarse crepitations in multiple lung fields. Severe pulmonary artery hypertension was noted on two-dimensional echocardiography. CT studies confirmed right pulmonary artery atresia with aspergilloma and bronchiectasis. With this diagnosis of UPAA, this case report intends to raise awareness among clinicians to consider this as a rare cause of cor pulmonale in an adult.

Etiology, characteristics and occurrence of heart diseases in rural Lesotho (ECHO-Lesotho): A retrospective echocardiography cohort study.

BACKGROUND: In 2019, 600'000 people in Africa died of heart failure and heart diseases will increase on the continent. It is crucial to understand the regional etiologies and risk factors for heart failure and underlying heart diseases. However, echocardiography data from rural Africa are scarce and from Lesotho non-existent. This study aims to examine the occurrence, characteristics and etiology of heart failure and heart diseases using echocardiography data from a referral hospital in rural Lesotho. METHODS: We conducted a retrospective cohort study at Seboche Mission Hospital, the only referral hospital in Butha-Buthe district (Lesotho) with an echocardiography department. We included data from all individuals referred to the department between January 2020 and May 2021. From non-hospitalized patients echocardiographic diagnosis, sex and age were available, from hospitalized patients additional sociodemographic and clinical data could be extracted. RESULTS: In the study period, a total of 352 echocardiograms were conducted; 213 had abnormal findings (among them 3 children). The majority of adult participants (130/210; 64%) were female and most frequent heart diseases were hypertensive (62/210, 30%), valvular (39/210, 19%) and chronic pulmonary (37/210, 18%). Heart failure represented 11% of hospitalizations in the same period. Among the 126 hospitalized heart failure patients, the most common etiology was chronic pulmonary heart disease (32/126; 25%). Former mine workers and people with a history of tuberculosis were more likely to have a chronic pulmonary heart disease. CONCLUSIONS: The leading cause of heart disease in this setting is hypertension. However, in contrast to other African epidemiological studies, chronic pulmonary heart disease is unexpectedly common. There is an urgent need to improve awareness and knowledge about lung diseases, make diagnostic and therapeutic options available and increase prevention.

Bedside critical ultrasound as a key to the diagnosis of obstructive atelectasis complicated with acute cor pulmonale and differentiation from pulmonary embolism: A case report.

Acute attack of dyspnea may be combined with acute cor pulmonale (ACP). Rapid and accurate identification of the etiology of ACP is the key to its diagnosis and treatment. Echocardiography is a better imaging tool in the assessment of right ventricular function. Under the guidance of the theory of cardiopulmonary interaction, ultrasonography can detect lung lesions, which causes ACP. We report the case of a 67-year-old man who received mechanical ventilation for acute respiratory failure. Right ventricular dysfunction was detected by echocardiography. Lung ultrasound showed a high risk of pulmonary embolism. However, obstructive atelectasis should not be ruled out after increasing back area ultrasonography. To avoid pitfalls, combined cardiac and lung ultrasound should be used carefully and strictly.

Usefulness of Right Ventricular Longitudinal Shortening Fraction to Detect Right Ventricular Dysfunction in Acute Cor Pulmonale Related to COVID-19.

OBJECTIVE: To compare two-dimensional-speckle tracking echocardiographic parameters (2D-STE) and classic echocardiographic parameters of right ventricular (RV) systolic function in patients with coronavirus disease 2019 (COVID-19)-related acute respiratory distress syndrome (CARDS) complicated or not by acute cor pulmonale (ACP). DESIGN: Prospective, between March 1, 2020 and April 15, 2020. SETTING: Intensive care unit of Amiens University Hospital (France). PARTICIPANTS: Adult patients with moderate-to-severe CARDS under mechanical ventilation for fewer than 24 hours. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Tricuspid annular displacement (TAD) parameters (TAD-septal, TAD-lateral, and RV longitudinal shortening fraction [RV-LSF]), RV global longitudinal strain (RV-GLS), and RV free wall longitudinal strain (RVFWLS) were measured using transesophageal echocardiography with a dedicated software and compared with classic RV systolic parameters (RV-FAC, S' wave, and tricuspid annular plane systolic excursion [TAPSE]). RV systolic dysfunction was defined as RV-FAC <35%. Twenty-nine consecutive patients with moderate-to-severe CARDS were included. ACP was diagnosed in 12 patients (41%). 2D-STE parameters were markedly altered in the ACP group, and no significant difference was found between patients with and without ACP for classic RV parameters (RV-FAC, S' wave, and TAPSE). In the ACP group, RV-LSF (17% [14%-22%]) had the best correlation with RV-FAC (r = 0.79, p < 0.001 v r = 0.27, p = 0.39 for RVGLS and r = 0.28, p = 0.39 for RVFWLS). A RV-LSF cut-off value of 17% had a sensitivity of 80% and a specificity of 86% to identify RV systolic dysfunction. CONCLUSIONS: Classic RV function parameters were not altered by ACP in patients with CARDS, contrary to 2D-STE parameters. RV-LSF seems to be a valuable parameter to detect early RV systolic dysfunction in CARDS patients with ACP.

Cor pulmonale: the role of traditional and advanced echocardiography in the acute and chronic settings.

Cor pulmonale is the condition in which the right ventricle undergoes morphological and/or functional changes due to diseases that affect the lungs, the pulmonary circulation, or the breathing process. Depending on the speed of onset of the pathological condition and subsequent effects on the right ventricle, it is possible to distinguish the acute cor pulmonale from the chronic type of disease. Echocardiography plays a central role in the diagnostic and therapeutic work-up of these patients, because of its non-invasive nature and wide accessibility, providing its greatest usefulness in the acute setting. It also represents a valuable tool for tracking right ventricular function in patients with cor pulmonale, assessing its stability, deterioration, or improvement during follow-up. In fact, not only it provides parameters with prognostic value, but also it can be used to assess the efficacy of treatment. This review attempts to provide the current standards of an echocardiographic evaluation in both acute and chronic cor pulmonale, focusing also on the findings present in the most common pathologies causing this condition.

Ecografía en el manejo del paciente crítico con infección por SARS-CoV-2 (COVID-19): aplicaciones clínicas en Medicina Intensiva-una revisión narrativa / Ultrasound in the Management of the Critically Ill Patient with SARS-CoV-2 Infection (COVID-19): clinical applications in Intensive Care Medicine -a narrative review

La infección por SARS-CoV-2 (COVID-19) se caracteriza por producir en las formas graves, un cuadro de insuficiencia respiratoria que puede evolucionar hacia neumonía y síndrome de distrés respiratorio agudo (SDRA), presentar complicaciones como fenómenos trombóticos y disfunción cardiaca, lo que motiva el ingreso en la Unidad de Cuidados Intensivos (UCI).La ecografía, convertida en una herramienta de uso habitual en la UCI, puede ser muy útil durante la pandemia COVID-19 ya que la información obtenida por el clínico puede ser interpretada e integrada en la valoración global durante la exploración del paciente. Este documento describe algunas de sus aplicaciones con el objetivo de proporcionar una guía a los médicos responsables adaptado al paciente crítico con COVID-19. Alguna de sus aplicaciones desde el ingreso en la UCI incluyen confirmar la correcta posición del tubo endotraqueal, facilitar la inserción segura de las vías, e identificar complicaciones y fenómenos trombóticos. Además, la ecografía pleuropulmonar puede ser una alternativa diagnóstica válida que permite evaluar el grado de afectación pulmonar, mediante el análisis de patrones ecográficos específicos, identificación del derrame pleural y del barotrauma. La ecocardiografía proporciona información acerca de la afectación cardíaca, detección del cor pulmonaley estados de shock

The clinical picture of SARS-CoV-2 infection (COVID-19) is characterized in its more severe form, by an acute respiratory failure which can worsen to pneumonia and acute respiratory distress syndrome (ARDS), and get complicated with thrombotic events and heart dysfunction. Therefore, admission to the Intensive Care Unit (ICU) is common.Ultrasound, which has become an everyday tool in the ICU, can be very useful during COVID-19 pandemic, since it provides the clinician with information which can be interpreted and integrated within a global assessment during the physical examination A description of some of the potential applications of ultrasound is depicted in this document, in order to supply the physicians taking care of these patients with a adapted guide to the intensive care setting.Some of its applications since ICU admission include verification of the correct position of the endotracheal tube, contribution to safe cannulation of lines, and identification of complications and thrombotic events. Furthermore, pleural and lung ultrasound can be an alternative diagnostic test to assess the degree of involvement of the lung parenchyma by means of the evaluation of specific ultrasound patterns, identification of pleural effusions and barotrauma. Echocardiography provides information of heart involvement, detects cor pulmonaleand shock states

[Ultrasound in the management of the critically ill patient with SARS-CoV-2 infection (COVID-19): narrative review]. / Ecografía en el manejo del paciente crítico con infección por SARS-CoV-2 (COVID-19): una revisión narrativa.

The clinical picture of SARS-CoV-2 infection (COVID-19) is characterized in its more severe form, by an acute respiratory failure which can worsen to pneumonia and acute respiratory distress syndrome (ARDS) and get complicated with thrombotic events and heart dysfunction. Therefore, admission to the Intensive Care Unit (ICU) is common. Ultrasound, which has become an everyday tool in the ICU, can be very useful during COVID-19 pandemic, since it provides the clinician with information which can be interpreted and integrated within a global assessment during the physical examination. A description of some of the potential applications of ultrasound is depicted in this document, in order to supply the physicians taking care of these patients with an adapted guide to the intensive care setting. Some of its applications since ICU admission include verification of the correct position of the endotracheal tube, contribution to safe cannulation of lines, and identification of complications and thrombotic events. Furthermore, pleural and lung ultrasound can be an alternative diagnostic test to assess the degree of involvement of the lung parenchyma by means of the evaluation of specific ultrasound patterns, identification of pleural effusions and barotrauma. Echocardiography provides information of heart involvement, detects cor pulmonale and shock states.

Novel Coronavirus-Induced Right Ventricular Failure and Point of Care Echocardiography: A Case Report.

Various cardiovascular complications have been reported in patients with coronavirus disease 2019. Common complications include acute myocardial injury, myocarditis, arrhythmia, pericarditis, heart failure, and shock. We present a case of cor pulmonale diagnosed with serial point of care ultrasound. Given the current shortage of personal protective equipment (PPE) and high infectivity of this virus, we acknowledge the utility of this tool in obtaining important clinical information while minimizing exposure and PPE consumption.

Arterial Vascular Pruning, Right Ventricular Size, and Clinical Outcomes in Chronic Obstructive Pulmonary Disease. A Longitudinal Observational Study.

Rationale: Cor pulmonale (right ventricular [RV] dilation) and cor pulmonale parvus (RV shrinkage) are both described in chronic obstructive pulmonary disease (COPD). The identification of emphysema as a shared risk factor suggests that additional disease characterization is needed to understand these widely divergent cardiac processes.Objectives: To explore the relationship between computed tomography measures of emphysema and distal pulmonary arterial morphology with RV volume, and their association with exercise capacity and mortality in ever-smokers with COPD enrolled in the COPDGene Study.Methods: Epicardial (myocardium and chamber) RV volume (RVEV), distal pulmonary arterial blood vessel volume (arterial BV5: vessels <5 mm2 in cross-section), and objective measures of emphysema were extracted from 3,506 COPDGene computed tomography scans. Multivariable linear and Cox regression models and the log-rank test were used to explore the association between emphysema, arterial BV5, and RVEV with exercise capacity (6-min-walk distance) and all-cause mortality.Measurements and Main Results: The RVEV was approximately 10% smaller in Global Initiative for Chronic Obstructive Lung Disease stage 4 versus stage 1 COPD (P < 0.0001). In multivariable modeling, a 10-ml decrease in arterial BV5 (pruning) was associated with a 1-ml increase in RVEV. For a given amount of emphysema, relative preservation of the arterial BV5 was associated with a smaller RVEV. An increased RVEV was associated with reduced 6-minute-walk distance and in those with arterial pruning an increased mortality.Conclusions: Pulmonary arterial pruning is associated with clinically significant increases in RV volume in smokers with COPD and is related to exercise capacity and mortality in COPD.Clinical trial registered with www.clinicaltrials.gov (NCT00608764).

A predominance of hypertensive heart disease among patients with cardiac disease in Buea, a semi-urban setting, South West Region of Cameroon.

OBJECTIVE: The pattern of heart disease is diverse within and among world regions. The little data on the spectrum of heart disease in Cameroon has been so far limited to major cities. We sought to describe the pattern of heart disease in Buea, the South West Region of Cameroon, a semi-urban setting. This was a descriptive cross-sectional study. Between June 2016 and April 2017 the echocardiography register of the Buea Regional Hospital was surveyed. We extracted data on the age, sex and echocardiographic diagnosis. RESULTS: Out of 529 patients who underwent echocardiography, 239 (45.2%) had a definite heart disease. There were 137 (57.3%) females. The mean age was 58 years (range 3-94 years). The most common echocardiographic diagnoses were hypertensive heart disease (43.2%), dilated cardiomyopathies (17.6%), ischemic heart diseases (9.6%), and cor pulmonale (8.8%). Rheumatic heart disease affected 6.7% of the patients. The most common rheumatic heart disease was mitral stenosis followed by mitral regurgitation. Congenital heart disease represented 2.1% and 5 patients (2.1%) had pulmonary hypertension. Hypertensive heart disease is the most common cardiac disease in this semi-urban region in Cameroon. Rheumatic heart disease still affects a sizable proportion of patients. Prevention of cardiac disease in our setting should focus on mass screening, the treatment and control of hypertension.

Correlation of right atrial enlargement on ECG to right atrial volume by echocardiography in patients with pulmonary hypertension.

Previous attempts to validate ECG criteria for right atrial (RA) enlargement (RAE) have been limited by sample sizes and lack of accepted standards for measuring RA size. New guidelines have recommended that RA volume (RAV) be used to determine RA size. Since these guidelines were released, no studies have been published that correlate RAE by ECG to RAV using the new standards. We aimed to validate previously proposed ECG criteria for RAE, commonly called P pulmonale, and to establish whether a correlation exists between P wave amplitudes and RAV as determined by echocardiogram in patients from the pulmonary hypertension (PHT) clinic. We identified patients from the PHT clinic that had an echocardiogram and ECG done at most 30days apart. We defined increased P wave amplitude as ≥2mm in lead II and ≥1mm in lead V1. The RA was determined to be enlarged if the RAV index (RAVI) was ≥39mL/m2 for men and ≥33mL/m2 for women. Patients were stratified into four groups: those with P II≥2mm, those with P V1≥1mm, those that met both criteria, and those that met neither. Right atrial volumes were then compared. Sixty-three patients were included in the study (7 men, 56 women). Three men and 36 women had an ECG that met criteria for P pulmonale. Five men and 28 women had an enlarged RA on echocardiogram. Sixty-nine percent of ECGs that met criteria for RAE were associated with increased RAV by echocardiogram. The specificity of each of the ECG criteria for P pulmonale at detecting RAE was 100% for men. The criterion with the highest specificity among women was P II≥2mm AND P V1≥1mm (94%). The least specific criterion for women was P II≥2mm (70%). The sensitivity of each criterion was much lower. The most sensitive criteria for men and women were P V1≥1mm (66.6%) and P II≥2mm (48%), respectively. The correlation of P wave amplitude in leads II and V1 and RAVI was not statistically significant for any of the ECG criteria for P pulmonale. In patients from the PHT clinic, the specificity of P pulmonale for detecting RAE is high, but the sensitivity is relatively low. These results suggest that in PHT, P pulmonale can be used to confirm that the RA is enlarged, but it is not a reliable test for diagnosing RAE.

Higher Plasma Myostatin Levels in Cor Pulmonale Secondary to Chronic Obstructive Pulmonary Disease.

OBJECTIVE: To analyze plasma myostatin levels and investigate their relationship with right ventricular (RV) function in patients with cor pulmonale secondary to chronic obstructive pulmonary disease (COPD). METHODS: The study recruited 81 patients with advanced COPD and 40 age-matched controls. The patients were divided into two groups: those with cor pulmonale and those without. Echocardiography was used to evaluate RV function and morphology, and the value of tricuspid annular plane systolic excursion (TAPSE) less than 16 mm was considered RV dysfunction. Plasma myostatin levels were analyzed by enzyme-linked immunosorbent assay, and B-type natriuretic peptide (BNP) levels were analyzed as a comparison of myostatin. RESULTS: The data detected cor pulmonale in 39/81 patients, with the mean value of TAPSE of 14.3 mm. Plasma myostatin levels (ng/mL) were significantly higher in patients with cor pulmonale (16.68 ± 2.95) than in those without (13.56 ± 3.09), and much higher than in controls (8.79±2.79), with each p<0.01. Significant differences were also found in plasma BNP levels among the three groups (p<0.05). Multivariate regression analysis suggested that myostatin levels were significantly correlated with the values of TAPSE and RV myocardium performance index among the COPD patients, and that BNP levels were significantly correlated only with systolic pulmonary arterial pressure, with each p<0.05. CONCLUSIONS: Plasma myostatin levels are increased in COPD patients who have cor pulmonale. Stronger correlations of plasma myostatin levels with echocardiographic indexes of the right heart suggest that myostatin might be superior to BNP in the early diagnosis of cor pulmonale in COPD.

Serum apelin as a novel non-invasive marker for subclinical cardiopulmonary complications in children and adolescents with sickle cell disease.

BACKGROUND: Cardiovascular involvement represents a leading cause of mortality and morbidity in sickle cell disease (SCD). Apelin is a peptide involved in the regulation of cardiovascular function. AIM: To determine serum apelin among 40 children and adolescents with SCD compared with 40 healthy controls and assess its relation to markers of hemolysis, iron overload as well as cardiopulmonary complications. METHODS: SCD patients, in steady state and asymptomatic for heart disease, were studied stressing on hydroxyurea/chelation therapy, hematological profile, serum ferritin and apelin levels. Full echocardiographic study including assessment of biventricular systolic function and pulmonary artery pressure was done. RESULTS: Apelin levels were significantly lower in SCD patients compared with controls (P<0.001). Cardiopulmonary complications were encountered in 30% of patients. Apelin was significantly decreased among patients with cardiopulmonary disease (P=0.006) whether those at risk of pulmonary hypertension (P=0.018) or patients with heart disease (P=0.043). Hydroxyurea-treated patients had higher apelin levels than untreated ones (P=0.001). Apelin was negatively correlated to lactate dehydrogenase, indirect bilirubin, serum ferritin, end systolic diameter, tricuspid regurgitant jet velocity, right ventricle systolic pressure, pulmonary vascular resistance and tissue Doppler imaging S wave. Apelin cutoff value of 1650ng/L could significantly detect the presence of cardiopulmonary complications in SCD with 90.9% sensitivity and 72.4% specificity. CONCLUSION: Apelin is a promising marker for screening of SCD patients at risk of cardiopulmonary disease because it is altered during the early subclinical stage of cardiac affection. A combination of apelin and echocardiography provides a reliable method to assess cardiopulmonary affection in young SCD patients.